Nineteen patients with hepatocellular carcinomaassociated with hepatolithiasis were retrospectivelyanalyzed. Eleven of the 19 patients presented withhepatolithiasis-related biliary infection. Diagnosis was erroneously assumed to be hepatolithiasisalone, liver abscess, or cholangiocarcinoma in five of11 patients before surgery was attempted. Middle-age,male sex, liver cirrhosis, hepatitis B or C infection, abnormal α-fetoprotein, and negativecarcinoembryonic antigen raised the suspicion ofassociated hepatocellular carcinoma rather thancholangiocarcinoma in patients with hepatolithiasis.Antibiotics and nonoperative methods to resolve biliaryinfection first, followed by hepatectomy, in selectedcases, to eradicate hepatocellular carcinoma andhepatolithiasis simultaneously provides the best chance for long-term survival. Otherwise, patientsoften died of hepatolithiasis-related biliary sepsisrather than hepatocellular carcinoma per se in the longrun.